Clinical characteristics and therapeutic efficacy analysis of primary bilateral macronodular adrenal hyperplasia

Abstract:

Objective To analyze the clinical characteristics and the efficacy of different surgical approaches of patients with primary bilateral macronodular adrenal hyperplasia（PBMAH）.Methods Clinical data of 119 patients diagnosed with adrenocorticotropic hormone（ACTH）-independent Cushing’s syndrome（CS） due to unilateral adrenal adenoma（UAA） and 34 patients with PBMAH in our hospital from January 2011 to August 2021 were analyzed retrospectively. Their clinical data such as clinical features，metabolic-related diseases，hormone secretion levels and imaging features were compared to summarize the clinical features of PBMAH. At the same time，the changes in clinical manifestations，metabolic diseases and hormone secretion levels of PBMAH patients before and after surgery were compared to to evaluate the therapeutic efficacy of different surgical procedures on PBMAH.Results Both PBMAH patients and UAA patients had significantly different clinical manifestations. The mean age at diagnosis was （49.8±13.1） years in the PBMAH group，which was significantly higher than that of the UAA group’s（43.0±11.6） years（P=0.016）. The incidence of PBMAH group was similar in both genderes，while the UAA group was significantly higher in women than in men（P<0.001）. Subclinical Cushing’s syndrome（SCS）（55.9%） was the main manifestation of PBMAH patients，and metabolic-related diseases such as hypertension and diabetes were the most common. In terms of hormone secretion levels，the cortisol level in the PBMAH group was significantly lower than that in the UAA group，especially serum cortisol at 16：00，24：00 and 24-hour urinary free cortisol. And 10 patients had a family history of genetic disorders，and were tested for mutations in the ARMC5 gene. Imaging showed diffuse large nodular hyperplasia of the adrenal glands bilaterally，whereas UAA patients had unilateral solitary adenomas. For treatment，PBMAH patients with SCS underwent unilateral adrenalectomy with a high clinical remission rate（100%），while PBMAH patients with Cushing’s appearance had a clinical remission rate of 50% after unilateral resection，and 16.7% of patients relapsed. And the incidence of adrenocortical insufficiency after unilateral adrenalectomy（28.6%） was significantly lower than that after bilateral adrenal surgery（100%）.Conclusion PBMAH is a familial hereditary disease with a higher age of onset，often manifesting as SCS，with hypertension and diabetes as the first symptoms. PBMAH patients combined with SCS are recommended to undertake unilateral total adrenalectomy. Patients with obvious symptoms of CS are recommended to undertake total adrenalectomy on one side and major adrenalectomy on the opposite side.